Successful Management of Severe Hypertriglyceridemia in a Neonate with Apolipoprotein A Deficiency: A Case Report with Literature Review

Abstract Introduction Reports regarding severe neonatal hypertriglyceridemia are scarce, and there is no consensus regarding its management. This report describes the successful management of a neonatal case of hypertriglyceridemia managed successfully. Case presentation A 2-day-old male neonate born to consanguineous parents presented with yellow skin discoloration. The mother had moderate hypertriglyceridemia. While testing for jaundice, the neonate’s blood was noticed to be milky. Blood lipid profile test showed highly elevated triglyceride (1,000-10,000 mg/dl), very-low-density lipoprotein level (1,800 mg/dl), and low-density lipoprotein (206 mg/dl) levels, and a low level of high-density lipoprotein (12 mg/dl). Further laboratory diagnosis revealed apolipoprotein A1 deficiency (39 mg/dl). The patient was put on a statin tablet of 0.25 mg twice daily and a formula diet (Monogen – Nutricia) low in triglycerides and rich in medium-chain triglycerides, and was given 1 ml Omega-3 syrup twice daily. After a dramatic decline in serum level, he was put on a 50% medium-chain-triglyceride formula and Gemfibrozil triglyceride-lowering agent. Conclusion Early diagnosis of severe hypertriglyceridemia in the neonatal period aids in the early initiation of treatment and prevention of severe complications. Conservative treatment via dietary modifications and supplementation can be associated with satisfactory outcomes.